手术治疗先天性束带综合征的疗效分析

Outcome evaluation of surgical treatment of congenital constriction band syndrome

目的 探讨先天性束带综合征（CCBS）的临床特点、治疗方法及预后情况.方法 2008年1月至2013年1月共收治先天性束带综合征患者19例（共95处缩窄带）,男9例,女10例;年龄3个月至19岁,平均73个月.26处Ⅰ度缩窄带采用一期“Z”字成形术进行松解;49处较窄的Ⅱ、Ⅲ度缩窄带采用二期“Z”字成形术予以松解;2处较宽的Ⅱ度缩窄带,则分二期手术切除缩窄带,再行带蒂皮瓣转移术予以修复;6处宫内截指（即Ⅳ度）行游离第二趾移植再造手指术;12处宫内截肢未予处理.术后采用Moses评估标准进行疗效评估.结果 手指与足趾缩窄带占全身的75.8％,上肢缩窄带发生率大于下肢,肢体远端缩窄带发生率大于肢体近端,长指（趾）比短指（趾）更易发病.手指、足趾主要为Ⅱ-Ⅳ度,前臂、上臂、小腿主要为Ⅰ度.本组病例平均随访30.5个月（12 - 60个月）.切口均Ⅰ期愈合,未见感染,皮瓣及肢体无坏死;肢体肿胀情况明显减退或消失,功能及外观得到明显改善,无复发病例.按Moses评估标准评定：优51处,良27处,差5处;优良率94％.结论 “Z”字成形术、皮瓣转移术及第二趾再造手指术能有效矫正CCBS患者肢体畸形,促进患肢的发育和功能的康复.

Objective To investigate the clinical characteristics,surgical approaches and prognosis of congenital constriction band syndrome.Methods We treated and followed up 19 patients （95 constriction bands） with congenital constriction band syndrome from January 2008 to January 2013.There were 9 boys and 10 girls.Their ages ranged from 3 months to 19 years with an average of 73 months.Twenty-six grade Ⅰ constriction rings were treated with one-stage Z-plasties.Two-stage Z-plasties were carried out in 49 narrow grade Ⅱ and grade Ⅲ constriction bands,with one-half of the band excised during the first operation and the other half excised half a year thereafter.Two wide grade Ⅱ constriction bands were excised by two-stage process and then the openings were covered by pedicled skin flaps.Six congenital amputations of the digits （grade Ⅳ） were treated with second toe transplantation to reconstruct the fingers.Twelve congenital amputations at any level of the extremities or digits were not treated.Moses criteria were used to evaluate the outcomes.Results 75.8 % of these constriction bands were located in the fingers or toes.The incidence of constriction bands was higher in the upper extremities than in the lower extremities and higher in the distal limb than in the proximal limb.Long fingers or toes were more likely to be affected than short fingers or toes.The constriction bands in the fingers and toes were mainly Hennigan grade Ⅱ to Ⅳ,while those in the arms,forearms and lower legs were mainly Hennigan grade Ⅰ.All the 19 cases were follow-up for an average of 30.5 months （range,12 to 60 months）.All the incisions healed primarily.There were no infections or necrosis of the limbs or flaps.Swelling of the extremities,fingers or toes was dramatically reduced or disappeared completely.There was no recurrence.The functions and appearances of the affected areas were significantly improved.According to Moses criteria the outcome was good at 51 sites,fair at 27 sites and poor at 5 sites.The satisfactory rate was 94%.Conclusion Z-plasties,flap transfer and finger reconstruction with second toe transplantation are effective to correct deformities,promote the development and functional rehabilitation of extremities in patients with congenital constriction band syndrome.